Gene: NPC1
Official Full Name: NPC intracellular cholesterol transporter 1provided by HGNC
Gene Summary: This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.[provided by RefSeq, Aug 2009]
Catalog Number | Product Name | Species | Gene | Passage ratio | Mycoplasma testing | Price |
---|---|---|---|---|---|---|
GP00510 | NPC1 gRNA3-gRNA4 KO plasmid | NPC1 | $850 | |||
KO00252 | NPC1 Knockout cell line (293T) | Human | NPC1 | 1:3~1:6 | Negative | Online Inquiry |
KO15681 | NPC1 Knockout cell line (HeLa) | Human | NPC1 | 1:3~1:6 | Negative | Online Inquiry |
KO15682 | NPC1 Knockout cell line (HCT 116) | Human | NPC1 | 1:2~1:4 | Negative | Online Inquiry |
KO15683 | NPC1 Knockout cell line (HEK293) | Human | NPC1 | 1:3~1:6 | Negative | Online Inquiry |
KO15684 | NPC1 Knockout cell line (A549) | Human | NPC1 | 1:3~1:4 | Negative | Online Inquiry |
NPC1 Gene Knockout Cell Lines are specialized cellular models created through targeted gene disruption of the NPC1 gene, which is crucial for the intracellular transport of cholesterol and other lipids. By eliminating the NPC1 gene, these cell lines enable researchers to study the pathophysiology of Niemann-Pick Disease type C and other lipid metabolism disorders. The knockout of the NPC1 gene suppresses its function, disrupting normal cholesterol trafficking and mimicking the cellular conditions associated with lipid-storage diseases.
The mechanisms underlying the functionality of NPC1 Gene Knockout Cell Lines are rooted in their ability to exhibit altered lipid metabolism, which can provide critical insights into disease mechanisms and potential therapeutic strategies. In these cells, researchers can observe accumulation of untransported cholesterol and gain a deeper understanding of cellular responses to lipid stress. This functional alteration allows for a more accurate assessment of therapeutic compounds aimed at restoring lipid homeostasis.
The scientific importance of NPC1 Gene Knockout Cell Lines is evident in both research and clinical settings, as they serve as an invaluable tool for drug discovery, genetic studies, and exploring gene therapy prospects. By mimicking the disease state in vitro, these cell lines facilitate the identification of small molecules or biologics that may correct the underlying defects associated with NPC1 dysfunction.
Unlike traditional cell lines that do not reflect the genetic pathology of Niemann-Pick Disease, NPC1 Gene Knockout Cell Lines offer specificity that is critical for reliable data and reproducibility in experimental results. The utilization of these tailored models can significantly enhance the relevance of findings, driving innovations within the field of lipid metabolism and genetic disorders.
For researchers and clinicians focused on deciphering the complexities of lipid metabolism and developing targeted therapies, NPC1 Gene Knockout Cell Lines represent a crucial asset. They empower scientists to explore novel therapeutic avenues while contributing to the broader understanding of metabolic diseases.
Our company is committed to providing cutting-edge biological products that support groundbreaking research. With expertise in cellular model development, we offer NPC1 Gene Knockout Cell Lines as part of our comprehensive portfolio, aimed at advancing scientific discovery and therapeutic development.
Please note that all services are for research use only. Not intended for any clinical use.
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